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学科主题临床医学
Clinical features and management of congenital long QT syndrome: a report on 54 patients from a national registry
Li, CL; Hu, DY; Qin, XG; Li, YT; Li, P; Liu, WL; Li, ZM; Li, L; Wang, LX
关键词long QT syndrome ECG QT interval beta-blocker cardiac electrophysiology
刊名HEART AND VESSELS
2004
DOI10.1007/s00380-003-0733-9
19期:1页:38-42
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Cardiac & Cardiovascular Systems ; Peripheral Vascular Disease
研究领域[WOS]Cardiovascular System & Cardiology
关键词[WOS]GENOTYPE-PHENOTYPE CORRELATION ; REPOLARIZATION ; MUTATIONS ; THERAPY ; HERG ; SPECTRUM ; BLOCKADE ; SCN5A ; GENES ; LQT1
英文摘要

To assess the clinical features and the management of congenital long QT syndrome (LQTS) in China, we collected the clinical data of 54 LQTS patients (14 males and 40 females) from our newly established national registry. All patients were symptomatic, with syncope being the most common symptom. The average age when the first symptoms occurred was 17.9+/-15.6 (range, 0.5-62) years; 55.6% of them had the first symptoms before the age of 20. The most common triggers of the symptoms were physical exercise or emotional stress. The average corrected QT interval was 0.55+/-0.08 s. Using ECG criteria, there were 14 (25.9%) LQT1 patients, 28 (51.9%) LQT2, and 2 (3.7%) LQT3. Thirty (55.6%) patients were treated with beta-blockers at the time of enrollment, with propranolol being the most commonly used drug, with an average daily dose of 57.5+/-39.1 mg. Four patients underwent left cardiac sympathectomy. After an average follow-up of 24.9+/-13.2 months, 3.1% (1/32) of patients with antiadrenergic therapy and 9.1% (2/22) without antiadrengergic therapy died of sudden cardiac death (P<0.05). We concluded that LQT2 might be the most common subtype in these patients. Antiadrenergic treatment was underused, raising the urgent need for educating both physicians and patients on the nature of the disease and its optimal antiadrenergic therapy.

语种英语
WOS记录号WOS:000187504300008
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被引频次:2[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/58501
专题北京大学第二临床医学院_心血管内科
作者单位1.Charles Sturt Univ, Sch Biomed Sci, Wagga Wagga, NSW 2678, Australia
2.Peking Univ, Peoples Hosp, Dept Cardiol, Beijing 100871, Peoples R China
推荐引用方式
GB/T 7714
Li, CL,Hu, DY,Qin, XG,et al. Clinical features and management of congenital long QT syndrome: a report on 54 patients from a national registry[J]. HEART AND VESSELS,2004,19(1):38-42.
APA Li, CL.,Hu, DY.,Qin, XG.,Li, YT.,Li, P.,...&Wang, LX.(2004).Clinical features and management of congenital long QT syndrome: a report on 54 patients from a national registry.HEART AND VESSELS,19(1),38-42.
MLA Li, CL,et al."Clinical features and management of congenital long QT syndrome: a report on 54 patients from a national registry".HEART AND VESSELS 19.1(2004):38-42.
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